Anaplastic large cell lymphoma overview: Difference between revisions
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==Overview== | ==Overview== | ||
Anaplastic large cell lymphoma is an aggressive (fast-growing) type of [[non-Hodgkin lymphoma]] that is usually of the T-cell type.<ref>NCI Dictionary of Cancer Terms. National Cancer Institute. http://www.cancer.gov/publications/dictionaries/cancer-terms?cdrid=45552 Accessed on October 6, 2015</ref> The anaplastic large cell lymphoma (ALCL) ALK-positive ('''A'''naplastic '''L'''lymphoma '''K'''inase) characterized by the proliferation of [[CD30]]-positive T-cells which have an abundant cytoplasm, a pleomorphic nucleus (horseshoe-shaped nucleus), and an eosinophilic paranuclear region.<ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693 }} </ref>ALK negative ALCL [[T-cell]]s express [[CD30]], but not the ALK ('''A'''naplastic '''L'''ymphoma '''K'''inase) chimeric protein,<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref> which explains the difference in clinical outcome compared to that of [[ALK(+)-ALCL]].<ref name="pmid25461779">{{cite journal| author=Xing X, Feldman AL| title=Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous. | journal=Adv Anat Pathol | year= 2015 | volume= 22 | issue= 1 | pages= 29-49 | pmid=25461779 | doi=10.1097/PAP.0000000000000047 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25461779 }} </ref>Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the [[peripheral T-cell lymphoma]] classification. Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype, clinical presentation, and histology. The ''ALK'' gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The ''DUSP22 '' gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear eosinophilic region are characteristic findings of anaplastic large cell lymphoma. There are no established causes for anaplastic large cell lymphoma. Anaplastic large cell lymphoma must be differentiated from other diseases such as [[metastatic]] [[carcinoma]], [[B cell lymphoma]], primary cutaneous T-cell lymphoma, [[rhabdomyosarcoma]], peripheral T-cell lymphoma-not otherwise specified, [[classical Hodgkin's lymphoma]], and [[diffuse large B cell lymphoma]]. Anaplastic large cell lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with anaplastic large cell lymphoma than females.<small><ref Name= Cancer.ca> Anaplastic large cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/anaplastic-large-cell-lymphoma/?region=on Accessed on October 7, 2015</ref></small>There are no established risk factors for anaplastic large cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for anaplastic large cell lymphoma. The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%. According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of anaplastic cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], chest pain, [[abdominal pain]], [[bone pain]], and painless swelling in the [[neck]], [[axilla]], [[groin]], [[thorax]], and [[abdomen]]. Common physical examination findings of anaplastic large cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref> Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015</ref>Laboratory tests for anaplastic large cell lymphoma include [[complete blood count]] (CBC), blood chemistry studies, [[flow cytometry]], [[FISH]], [[immunohistochemistry]], and [[immunophenotyping]]. Chest X ray may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and pleural effusion. Thorax CT scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]] and bilateral [[pleural effusion]]. Thorax MRI scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and [[lymphadenopathy]]. Lymph node or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma. Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma. [[PET]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Other diagnostic studies for anaplastic large cell lymphoma include [[laparoscopy]], [[laparotomy]], [[bone marrow aspiration]], and [[bone marrow biopsy]]. The predominant therapy for anaplastic lymphoma is [[chemotherapy]]. Adjunctive [[radiotherapy]], [[stem cell transplantation]], and [[surgery]] may be required. The optimal therapy for anaplastic large cell lymphoma depends on the type and extent of anaplastic large cell lymphoma. The feasibility of surgery depends on the type of anaplastic large cell lymphoma. | Anaplastic large cell lymphoma is an aggressive (fast-growing) type of [[non-Hodgkin lymphoma]] that is usually of the [[T-cell]] type.<ref>NCI Dictionary of Cancer Terms. National Cancer Institute. http://www.cancer.gov/publications/dictionaries/cancer-terms?cdrid=45552 Accessed on October 6, 2015</ref> The anaplastic large cell lymphoma (ALCL) ALK-positive ('''A'''naplastic '''L'''lymphoma '''K'''inase) characterized by the proliferation of [[CD30]]-positive T-cells which have an abundant cytoplasm, a [[pleomorphic]] nucleus (horseshoe-shaped nucleus), and an [[eosinophilic]] paranuclear region.<ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693 }} </ref>ALK negative ALCL [[T-cell]]s express [[CD30]], but not the ALK ('''A'''naplastic '''L'''ymphoma '''K'''inase) chimeric protein,<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref> which explains the difference in clinical outcome compared to that of [[ALK(+)-ALCL]].<ref name="pmid25461779">{{cite journal| author=Xing X, Feldman AL| title=Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous. | journal=Adv Anat Pathol | year= 2015 | volume= 22 | issue= 1 | pages= 29-49 | pmid=25461779 | doi=10.1097/PAP.0000000000000047 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25461779 }} </ref>Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization ([[WHO]]) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the [[peripheral T-cell lymphoma]] classification. Anaplastic large cell lymphoma may be classified into several subtypes based on [[immunophenotype]], clinical presentation, and [[histology]]. The ''ALK'' gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The ''DUSP22 '' gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear [[eosinophilic]] region are characteristic findings of anaplastic large cell lymphoma. There are no established causes for anaplastic large cell lymphoma. Anaplastic large cell lymphoma must be differentiated from other diseases such as [[metastatic]] [[carcinoma]], [[B cell lymphoma]], primary cutaneous T-cell lymphoma, [[rhabdomyosarcoma]], peripheral T-cell lymphoma-not otherwise specified, [[classical Hodgkin's lymphoma]], and [[diffuse large B cell lymphoma]]. Anaplastic large cell lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with anaplastic large cell lymphoma than females.<small><ref Name= Cancer.ca> Anaplastic large cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/anaplastic-large-cell-lymphoma/?region=on Accessed on October 7, 2015</ref></small>There are no established risk factors for anaplastic large cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for anaplastic large cell lymphoma. The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%. According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of anaplastic cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], chest pain, [[abdominal pain]], [[bone pain]], and painless swelling in the [[neck]], [[axilla]], [[groin]], [[thorax]], and [[abdomen]]. Common physical examination findings of anaplastic large cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref> Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015</ref>Laboratory tests for anaplastic large cell lymphoma include [[complete blood count]] (CBC), blood chemistry studies, [[flow cytometry]], [[FISH]], [[immunohistochemistry]], and [[immunophenotyping]]. [[Chest X ray]] may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and [[pleural effusion]]. Thorax [[CT]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]] and bilateral [[pleural effusion]]. Thorax [[MRI]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on [[MRI]] scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and [[lymphadenopathy]]. [[Lymph node]] or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma. Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma. [[PET]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Other diagnostic studies for anaplastic large cell lymphoma include [[laparoscopy]], [[laparotomy]], [[bone marrow aspiration]], and [[bone marrow biopsy]]. The predominant therapy for anaplastic lymphoma is [[chemotherapy]]. Adjunctive [[radiotherapy]], [[stem cell transplantation]], and [[surgery]] may be required. The optimal therapy for anaplastic large cell lymphoma depends on the type and extent of anaplastic large cell lymphoma. The feasibility of surgery depends on the type of anaplastic large cell lymphoma. | ||
==Historical Perspective== | ==Historical Perspective== | ||
Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the [[peripheral T-cell lymphoma]] classification. | Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the [[peripheral T-cell lymphoma]] classification. | ||
==Classification== | ==Classification== | ||
Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype, clinical presentation, and histology. | Anaplastic large cell lymphoma may be classified into several subtypes based on [[immunophenotype]], clinical presentation, and [[histology]]. | ||
==Pathophysiology== | ==Pathophysiology== | ||
The ''ALK'' gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The ''DUSP22 '' gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear eosinophilic region are characteristic findings of anaplastic large cell lymphoma. | The ''ALK'' gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The ''DUSP22 '' gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear [[eosinophilic]] region are characteristic findings of anaplastic large cell lymphoma. | ||
==Causes== | ==Causes== | ||
There are no established causes for anaplastic large cell lymphoma. | There are no established causes for anaplastic large cell lymphoma. | ||
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According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement. | According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement. | ||
===Symptoms=== | ===Symptoms=== | ||
The most common symptoms of anaplastic cell lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], chest pain, [[abdominal pain]], [[bone pain]], and painless swelling in the neck, axilla, groin, thorax, and abdomen. | The most common symptoms of anaplastic cell lymphoma include [[fever]], [[weight loss]], [[skin rash]], [[night sweats]], [[chest pain]], [[abdominal pain]], [[bone pain]], and painless swelling in the [[neck]], [[axilla]], [[groin]], [[thorax]], and [[abdomen]]. | ||
===Physical examination=== | ===Physical examination=== | ||
Common physical examination findings of mantle cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref> Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015</ref> | Common physical examination findings of mantle cell lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], [[hepatomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref> Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015</ref> | ||
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Laboratory tests for anaplastic large cell lymphoma include [[complete blood count]] (CBC), blood chemistry studies, [[flow cytometry]], [[FISH]], [[immunohistochemistry]], and [[immunophenotyping]].<ref name="ALK+/ALK-">{{cite web|url=http://www.bloodjournal.org/content/111/12/5496?sso-checked=true|title=ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}}</ref><ref name = hindawi> Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma: An Underrecognized Aggressive Lymphoma. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2012/529572/#B1 Accessed on October 13, 2015</ref><ref name = dovepress>Dovepress. Anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma with involvement of the urinary bladder: a case report and review of literature. https://www.dovepress.com/anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-wit-peer-reviewed-article-OTT Accessed on October 13, 2015</ref> | Laboratory tests for anaplastic large cell lymphoma include [[complete blood count]] (CBC), blood chemistry studies, [[flow cytometry]], [[FISH]], [[immunohistochemistry]], and [[immunophenotyping]].<ref name="ALK+/ALK-">{{cite web|url=http://www.bloodjournal.org/content/111/12/5496?sso-checked=true|title=ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project}}</ref><ref name = hindawi> Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma: An Underrecognized Aggressive Lymphoma. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2012/529572/#B1 Accessed on October 13, 2015</ref><ref name = dovepress>Dovepress. Anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma with involvement of the urinary bladder: a case report and review of literature. https://www.dovepress.com/anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-wit-peer-reviewed-article-OTT Accessed on October 13, 2015</ref> | ||
===Chest X ray=== | ===Chest X ray=== | ||
Chest X ray may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and pleural effusion. | [[Chest X ray]] may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and [[pleural effusion]]. | ||
===CT Scan=== | ===CT Scan=== | ||
Thorax CT scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]] and bilateral [[pleural effusion]]. | Thorax [[CT]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include [[Lymphadenopathy|mediastinal lymphadenopathy]] and bilateral [[pleural effusion]]. | ||
===MRI Scan=== | ===MRI Scan=== | ||
Thorax MRI scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and [[lymphadenopathy]]. | Thorax [[MRI]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and [[lymphadenopathy]]. | ||
===Biopsy=== | ===Biopsy=== | ||
Lymph node or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma. | [[Lymph node]] or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma. | ||
===Ultrasound=== | ===Ultrasound=== | ||
Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma. | Abdomen and shoulder [[ultrasound]] may be helpful in the diagnosis of anaplastic large cell lymphoma. | ||
===Other Imaging Studies=== | ===Other Imaging Studies=== | ||
[[PET]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. | [[PET]] scan may be helpful in the diagnosis of anaplastic large cell lymphoma. | ||
Revision as of 16:31, 16 November 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
Anaplastic large cell lymphoma is an aggressive (fast-growing) type of non-Hodgkin lymphoma that is usually of the T-cell type.[1] The anaplastic large cell lymphoma (ALCL) ALK-positive (Anaplastic Llymphoma Kinase) characterized by the proliferation of CD30-positive T-cells which have an abundant cytoplasm, a pleomorphic nucleus (horseshoe-shaped nucleus), and an eosinophilic paranuclear region.[2]ALK negative ALCL T-cells express CD30, but not the ALK (Anaplastic Lymphoma Kinase) chimeric protein,[3] which explains the difference in clinical outcome compared to that of ALK(+)-ALCL.[4]Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the peripheral T-cell lymphoma classification. Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype, clinical presentation, and histology. The ALK gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The DUSP22 gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear eosinophilic region are characteristic findings of anaplastic large cell lymphoma. There are no established causes for anaplastic large cell lymphoma. Anaplastic large cell lymphoma must be differentiated from other diseases such as metastatic carcinoma, B cell lymphoma, primary cutaneous T-cell lymphoma, rhabdomyosarcoma, peripheral T-cell lymphoma-not otherwise specified, classical Hodgkin's lymphoma, and diffuse large B cell lymphoma. Anaplastic large cell lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with anaplastic large cell lymphoma than females.[5]There are no established risk factors for anaplastic large cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for anaplastic large cell lymphoma. The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%. According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of anaplastic cell lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of anaplastic large cell lymphoma include fever, rash, ulcer, splenomegaly, hepatomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[6]Laboratory tests for anaplastic large cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, FISH, immunohistochemistry, and immunophenotyping. Chest X ray may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and pleural effusion. Thorax CT scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include mediastinal lymphadenopathy and bilateral pleural effusion. Thorax MRI scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and lymphadenopathy. Lymph node or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma. Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma. PET scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Other diagnostic studies for anaplastic large cell lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy. The predominant therapy for anaplastic lymphoma is chemotherapy. Adjunctive radiotherapy, stem cell transplantation, and surgery may be required. The optimal therapy for anaplastic large cell lymphoma depends on the type and extent of anaplastic large cell lymphoma. The feasibility of surgery depends on the type of anaplastic large cell lymphoma.
Historical Perspective
Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the peripheral T-cell lymphoma classification.
Classification
Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype, clinical presentation, and histology.
Pathophysiology
The ALK gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The DUSP22 gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear eosinophilic region are characteristic findings of anaplastic large cell lymphoma.
Causes
There are no established causes for anaplastic large cell lymphoma.
Differential Diagnosis
Anaplastic large cell lymphoma must be differentiated from other diseases such as metastatic carcinoma, B cell lymphoma, primary cutaneous T-cell lymphoma, rhabdomyosarcoma, peripheral T-cell lymphoma-not otherwise specified, classical Hodgkin's lymphoma, and diffuse large B cell lymphoma.
Epidemiology and Demographics
Anaplastic large cell lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with anaplastic large cell lymphoma than females.[5]
Risk Factors
There are no established risk factors for anaplastic large cell lymphoma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for anaplastic large cell lymphoma.
Prognosis
The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%.
Diagnosis
Staging
According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement.
Symptoms
The most common symptoms of anaplastic cell lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.
Physical examination
Common physical examination findings of mantle cell lymphoma include fever, rash, ulcer, splenomegaly, hepatomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[7]
Laboratory tests
Laboratory tests for anaplastic large cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, FISH, immunohistochemistry, and immunophenotyping.[8][9][10]
Chest X ray
Chest X ray may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and pleural effusion.
CT Scan
Thorax CT scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include mediastinal lymphadenopathy and bilateral pleural effusion.
MRI Scan
Thorax MRI scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and lymphadenopathy.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma.
Ultrasound
Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma.
Other Imaging Studies
PET scan may be helpful in the diagnosis of anaplastic large cell lymphoma.
Other Diagnostic Studies
Other diagnostic studies for anaplastic large cell lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy.
Treatment
Medical Therapy
The predominant therapy for anaplastic lymphoma is chemotherapy. Adjunctive radiotherapy, stem cell transplantation, and surgery may be required. The optimal therapy for anaplastic large cell lymphoma depends on the type and extent of anaplastic large cell lymphoma.
Surgery
The feasibility of surgery depends on the type of anaplastic large cell lymphoma.
References
- ↑ NCI Dictionary of Cancer Terms. National Cancer Institute. http://www.cancer.gov/publications/dictionaries/cancer-terms?cdrid=45552 Accessed on October 6, 2015
- ↑ Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ; et al. (1998). "ALK-positive lymphoma: a single disease with a broad spectrum of morphology". Blood. 91 (6): 2076–84. PMID 9490693.
- ↑ Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
- ↑ Xing X, Feldman AL (2015). "Anaplastic large cell lymphomas: ALK positive, ALK negative, and primary cutaneous". Adv Anat Pathol. 22 (1): 29–49. doi:10.1097/PAP.0000000000000047. PMID 25461779.
- ↑ 5.0 5.1 Anaplastic large cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/anaplastic-large-cell-lymphoma/?region=on Accessed on October 7, 2015
- ↑ Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015
- ↑ Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015
- ↑ "ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project".
- ↑ Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma: An Underrecognized Aggressive Lymphoma. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2012/529572/#B1 Accessed on October 13, 2015
- ↑ Dovepress. Anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma with involvement of the urinary bladder: a case report and review of literature. https://www.dovepress.com/anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-wit-peer-reviewed-article-OTT Accessed on October 13, 2015